Organic acidaemia and Hyperammonaemia: review.
Recent clinical and experimental evidence on the effects of organic acids in producing or ameliorating hyperammonaemia is reviewed. The importance of hepatic mitochondrial N-acetylglutamate and its precursors, glutamate and acetyl-CoA, in the control of ureagenesis and thus blood ammonia levels is emphasized by recent work. The hypothesis is proposed that protein loads stimulated urea cycle activity via glutamate-induced changes in N-acetylglutamate concentration, while the effects of organic acids on ureagenesis are related in a predictable way to their effects on hepatic concentrations of acetyl-CoA.
D000105:Acetyl Coenzyme A / Q000378:metabolism; D000641:Ammonia / Q000097:blood / Q000378:metabolism*; D000818:Animals; D002222:Carbamoyl-Phosphate Synthase (Ammonia) / Q000378:metabolism; D002264:Carboxylic Acids / Q000378:metabolism*; D003065:Coenzyme A / Q000378:metabolism; D005971:Glutamates / Q000378:metabolism; D006801:Humans; D008661:Metabolism, Inborn Errors / Q000378:metabolism*; D008930:Mitochondria, Liver / Q000378:metabolism; D051381:Rats
D002264:Carboxylic Acids; D005971:Glutamates; D000105:Acetyl Coenzyme A; D000641:Ammonia; D002222:Carbamoyl-Phosphate Synthase (Ammonia); C016195:N-acetylglutamic acid; D003065:Coenzyme A